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  • 1995-1999  (1)
  • 1985-1989  (1)
  • (adenylate-, AMP-)deaminase deficiency  (1)
  • Datensicherheit  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 67 (1989), S. 1205-1213 
    ISSN: 1432-1440
    Keywords: (D-)ribose ; Metabolism ; Myoadenylate ; (adenylate-, AMP-)deaminase deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary D-ribose was administered orally or intravenously over at least 5 h to eight healthy volunteers and five patients with myoadenylate deaminase deficiency. Intravenous administration rates were 83, 167, and 222 mg/kg/h, which were well tolerated but oral administration of more than 200 mg/kg/h caused diarrhea. The average steady state serum ribose level ranged between 4.8 mg/100 ml (83 mg/kg/h, oral administration) and 81.7 mg/100 ml (222 mg/kg/h, intravenous administration). Serum glucose level decreased during ribose administration. The intestinal absorption rate of orally administered ribose was 87.8%–99.8% of the intake at doses up to 200 mg/kg/h without first pass effect. Urinary losses were 23% of the intravenously administered dose at 222 mg/kg/h. Ribose appeared to be excreted by glomerular filtration without active reabsorption; a renal threshold could not be demonstrated. The amount of ribose transported back from the tubular lumen depended on the serum ribose level. There was no difference in ribose turnover in healthy subjects and patients with MAD deficiency.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1433-0458
    Keywords: Schlüsselwörter Datenschutz ; Datensicherheit ; Medizinische Register ; Datenerhebung ; Kindliche Hörstörungen ; Key words Medical registries ; Data collection and security ; Hearing loss and childhood
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The German Registry for Hearing Loss in Children (DZH) processes nationwide data from audiological centers. Coping with the accrued data and its subsequent management and analysis requires a high degree of security and control. To establish a nationwide registry it is necessary at an early stage to take into consideration the legal requirements of the participating states. Use of the DZH as an example demonstrates how a pragmatic solution can be reached. Special issues concerning data collection, transfer, storage and deletion, coding strategies to ensure anonymity, checking for duplicate entries, data separation, and automated data analysis and data protection are explained.
    Notes: Zusammenfassung Das Deutsche Zentralregister für kindliche Hörstörungen (DZH) verarbeitet bundesweit Daten von verschiedenen audiologischen Einrichtungen. Die Bewältigung der anfallenden Datenmengen, die nachfolgende Datenverwaltung und -analyse erfordern neben einer differenzierten und kontrollierbaren Verarbeitung ein Höchstmaß an Datensicherheit. Vor allem die länderübergreifende Struktur eines Registers erfordert schon bei der Planung engste Zusammenarbeit mit dem zuständigen Landesdatenschutzbeauftragten und auch mit den Landesdatenschutzbeauftragten anderer beteiligter Bundesländer. Am Beispiel des DZH wird demonstriert, wie eine kooperative Zusammenarbeit pragmatisch realisiert werden kann. Besonderheiten bei der Datenerhebung, Datentransfer, Speicherung und Löschung von Daten, technische Datenschutzmaßnahmen, Sicherstellung von Anonymität durch Codierungsstrategien, Duplikatsprüfung, Datentrennung und automatisierte Datenauswertung werden an Beispielen erläutert.
    Type of Medium: Electronic Resource
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